Introduction: Resistance to treatments is a common feature of Ohtahara, Aicardi, West and Dravet syndromes, as well as malignant migrating epilepsy in infancy.
Aims: To update the therapeutic management and to analyse whether the aetiology somehow determines the treatment.
Development: Convulsive seizures in the first year of life may be due to a potentially treatable aetiology, which makes it essential to carry out a complete evaluation so as to be able to begin, as early as possible, the most suitable and the non-specific symptomatic treatments to control the seizures, which prevents or minimises their deleterious effects. Metabolic disease must be ruled out and it is also essential to try a therapeutic regimen of vitamins and cofactors, as well as antiepileptic drugs. In Ohtahara and Aicardi syndromes, the first-order treatment is phenobarbital and phenytoin, and the most commonly used second-order drugs are midazolam, levetiracetam, lidocaine and valproate. In West's syndrome, the first-order treatment consists of adrenocorticotropic hormone and vigabatrine for the case of tuberous sclerosis; if there is no response, other pharmaceuticals, a ketogenic diet and surgery must be considered. For Dravet's syndrome, the main treatment consists in valproate with clobazam and stiripentol, and as the second order, other drugs and a ketogenic diet should be considered. In epilepsy with migrating seizures, the most effective treatment is with bromides, stiripentol, clonazepam and levetiracetam.
Conclusions: Today there is little consensus on the therapeutic approach to be able to establish specific indications. The aetiology has an influence on the treatment, both in cases in which a curative treatment exists (metabolic diseases) and in the symptomatic management with antiepileptic drugs or other treatments (ketogenic diet or surgery).
Title: Encefalopatias epilepticas del lactante. Como las tratamos? Influye la etiologia en la respuesta al tratamiento?
Introduccion. La refractariedad es una caracteristica comun del tratamiento de los sindromes de Ohtahara, Aicardi, West, Dravet y epilepsia maligna del lactante con crisis migrantes. Objetivo. Actualizar el manejo terapeutico y analizar si la etiologia determina de alguna manera el tratamiento. Desarrollo. Las crisis convulsivas en el primer año de vida pueden deberse a una etiologia potencialmente tratable, por lo que es imperativo una completa evaluacion para instaurar de manera precoz el tratamiento adecuado y el sintomatico no especifico para el control de las crisis, que evite o minimice el efecto deletereo de estas. Es obligado hasta descartar metabolopatia y ensayar pauta de vitaminas y cofactores, ademas de antiepilepticos. En los sindromes de Ohtahara y Aicardi, la primera linea es fenobarbital y fenitoina, y en segunda linea, los mas habituales son midazolam, levetiracetam, lidocaina y valproato. En el sindrome de West, la primera linea la constituye la hormona adrenocorticotropa y la vigabatrina para el caso de esclerosis tuberosa; si no hay respuesta, considerar otros farmacos, dieta cetogenica y cirugia. Para el sindrome de Dravet, los principales son valproato con clobazam y estiripentol, y de segunda linea, considerar otros farmacos y dieta cetogenica. En la epilepsia con crisis migrantes, los mas eficaces son bromuros, estiripentol, clonacepam y levetiracetam. Conclusiones. Actualmente existe poco consenso en el abordaje terapeutico para establecer indicaciones taxativas. La etiologia influye en el tratamiento, tanto en el caso de disponer de tratamiento curativo (metabolopatias) como en el abordaje sintomatico con antiepilepticos u otros tratamientos (dieta cetogenica o cirugia).